Human Metabolome Database Version 3.5

Showing metabocard for Succinic acid (HMDB00254)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-05-29 13:25:16 -0600
HMDB ID HMDB00254
Secondary Accession Numbers None
Metabolite Identification
Common Name Succinic acid
Description Succinic acid is a dicarboxylic acid. The anion, succinate, is a component of the citric acid cycle capable of donating electrons to the electron transfer chain. Succinate dehydrogenase (SDH) plays an important role in the mitochondria, being both part of the respiratory chain and the Krebs cycle. SDH with a covalently attached FAD prosthetic group, binds enzyme substrates (succinate and fumarate) and physiological regulators (oxaloacetate and ATP). Oxidizing succinate links SDH to the fast-cycling Krebs cycle portion where it participates in the breakdown of acetyl-CoA throughout the whole Krebs cycle. The succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate. (PMID 16143825 Link_out) Mutations in the four genes encoding the subunits of the mitochondrial respiratory chain succinate dehydrogenase are associated with a wide spectrum of clinical presentations (i.e.: Huntington's disease. (PMID 11803021 Link_out).
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. 1,2-Ethanedicarboxylate
  2. 1,2-Ethanedicarboxylic acid
  3. 1,4-Butanedioate
  4. 1,4-Butanedioic acid
  5. Amber acid
  6. Asuccin
  7. Dihydrofumarate
  8. Dihydrofumaric acid
  9. Katasuccin
  10. Succinate
  11. Wormwood acid
Chemical Formula C4H6O4
Average Molecular Weight 118.088
Monoisotopic Molecular Weight 118.02660868
IUPAC Name butanedioic acid
Traditional IUPAC Name succinic acid
CAS Registry Number 110-15-6
SMILES OC(=O)CCC(O)=O
InChI Identifier InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)
InChI Key KDYFGRWQOYBRFD-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Organic Acids and Derivatives
Class Carboxylic Acids and Derivatives
Sub Class Dicarboxylic Acids and Derivatives
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Dicarboxylic acids(Lipidmaps)
  • Fatty Acids and Conjugates
  • alpha,omega-dicarboxylic acid(ChEBI)
Substituents
  • Carboxylic Acid
Direct Parent Dicarboxylic Acids and Derivatives
Ontology
Status Detected and Quantified
Origin
  • Endogenous
  • Microbial
Biofunction
  • Component of Arginine and proline metabolism
  • Component of Butanoate metabolism
  • Component of C5-Branched dibasic acid metabolism
  • Component of Glutamate metabolism
  • Component of Propanoate metabolism
  • DNA component
Application Not Available
Cellular locations
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 185 - 188 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 83.2 mg/mL Not Available
LogP -0.59 HANSCH,C ET AL. (1995)
Predicted Properties
Property Value Source
Water Solubility 211 g/L ALOGPS
LogP -0.53 ALOGPS
LogP -0.4 ChemAxon
LogS 0.25 ALOGPS
pKa (strongest acidic) 3.55 ChemAxon
Hydrogen Acceptor Count 4 ChemAxon
Hydrogen Donor Count 2 ChemAxon
Polar Surface Area 74.6 A2 ChemAxon
Rotatable Bond Count 3 ChemAxon
Refractivity 23.54 ChemAxon
Polarizability 10.16 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -2 ChemAxon
Spectra
Gas-MS Spectrum
13C NMR Spectrum
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum EI-B (Unknown)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic)
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 40
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 50
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
[1H,1H] 2D NMR Spectrum
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Muscle
  • Skeletal Muscle
  • Fibroblasts
  • Pancreas
  • Placenta
  • Kidney
  • Liver
  • Brain
  • Prostate
  • Adipose Tissue
  • Spleen
Pathways
Name SMPDB Link KEGG Link
Citric Acid Cycle SMP00057 map00020 Link_out
Glutamate Metabolism SMP00072 map00250 Link_out
Mitochondrial Electron Transport Chain SMP00355 map00190 Link_out
Phytanic Acid Peroxisomal Oxidation SMP00450 Not Available
Carnitine Synthesis SMP00465 Not Available
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified
16.0 (0.00-32.0) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
23.5 uM Adult (>18 years old) Not Specified Normal
Blood Detected and Quantified
8.8 +/- 2.7 uM Adult (>18 years old) Both Normal
  • Geigy Scient...
Cerebrospinal Fluid (CSF) Detected and Quantified
47.2 uM Adult (>18 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
19 uM Adult (>18 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
150.0 +/- 110.0 uM Elderly (>65 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
3 +/- 2 uM Not Specified Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
28.5 (24-33) uM Adult (>18 years old) Both Normal
  • Geigy Scient...
Cerebrospinal Fluid (CSF) Detected and Quantified
3.0 +/- 2.0 uM Adult (>18 years old) Both Normal
Saliva Detected and Quantified
2260 (60.0-4460) uM Adult (>18 years old) Both Normal
Saliva Detected and Quantified
4-145 uM Adult (>18 years old) Male Comment normal
Saliva Detected and Quantified
1-33 uM Adult (>18 years old) Male Comment normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Normal
Urine Detected and Quantified
4.7 (1.1-14.5) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
12.60 +/- 12.13 umol/mmol creatinine Infant (0-1 year old) Both Normal
Urine Detected and Quantified
14.48 +/- 3.20 umol/mmol creatinine Children (1-13 year old) Both Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
7.7 (1.9-20.0) umol/mmol creatinine Adolescent (13-18 years old) Both Normal
Urine Detected and Quantified
3.8 (1.25-6.7) umol/mmol creatinine Adult (>18 years old) Both Normal
  • Geigy Scient...
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Both Normal
Urine Detected and Quantified
2.10 umol/mmol creatinine Adult (>18 years old) Both Normal
  • Shaykhutdino...
Urine Detected and Quantified
9.9 +/- 5.0 umol/mmol creatinine Adult (>18 years old) Male Normal
Urine Detected and Quantified
14.4 +/- 4.9 umol/mmol creatinine Adult (>18 years old) Female Normal
Urine Detected and Quantified
7.5 (0.5-16) umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
6.0 (0.3-33.3) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Comment Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Comment Normal
Urine Detected and Quantified
5.6 +/- 3.8 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
197.2 (29.4-486.2) umol/mmol creatinine Newborn (0-30 days old) Both Normal
Urine Detected and Quantified
185.4 (6.0-342.6) umol/mmol creatinine Infant (0-1 year old) Both Normal
Urine Detected and Quantified
11.6 (4.0-27.3) umol/mmol creatinine Children (1-13 year old) Both Normal
Urine Detected and Quantified
8.25 (0.5-16.0) umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
1.3 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
6.2 (2.5-13.5) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Cerebrospinal Fluid (CSF) Detected and Quantified 19.0 (0.0-38.0) uM Adult (>18 years old) Both Canavan Disease
Cerebrospinal Fluid (CSF) Detected and Quantified 55.0 (19.0-91.0) uM Adult (>18 years old) Both Alzheimer's disease
Urine Detected and Quantified 43.35 +/- 25.08 umol/mmol creatinine Children (1-13 year old) Both Severely malnourished children
Urine Detected and Quantified 9.0 +/- 6.0 umol/mmol creatinine Adult (>18 years old) Both Lung cancer
Associated Disorders and Diseases
Disease References
Canavan disease
  • Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. Pubmed: 7729054 Link_out
      Alzheimer's disease
      • Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. Pubmed: 9693263 Link_out
          Lung Cancer
          • Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2008 Oct 25. Pubmed: 18953024 Link_out
              Associated OMIM IDs
              DrugBank ID DB00139 Link_out
              DrugBank Metabolite ID Not Available
              Phenol Explorer Compound ID Not Available
              Phenol Explorer Metabolite ID Not Available
              FoodDB ID FDB001931
              KNApSAcK ID C00001205 Link_out
              Chemspider ID 1078 Link_out
              KEGG Compound ID C00042 Link_out
              BioCyc ID SUC Link_out
              BiGG ID 33633 Link_out
              Wikipedia Link Succinic acid Link_out
              NuGOwiki Link HMDB00254 Link_out
              Metagene Link HMDB00254 Link_out
              METLIN ID 114 Link_out
              PubChem Compound 1110 Link_out
              PDB ID SIN Link_out
              ChEBI ID 15741 Link_out
              References
              Synthesis Reference Berglund, Kris Arvid; Andersson, Christian; Rova, Ulrika. Process for the production of succinic acid. PCT Int. Appl. (2007), 30pp.
              Material Safety Data Sheet (MSDS) Download (PDF)
              General References
              1. Magera MJ, Helgeson JK, Matern D, Rinaldo P: Methylmalonic acid measured in plasma and urine by stable-isotope dilution and electrospray tandem mass spectrometry. Clin Chem. 2000 Nov;46(11):1804-10. Pubmed: 11067816 Link_out
              2. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. Pubmed: 8087979 Link_out
              3. Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. Pubmed: 9693263 Link_out
              4. Zhang TM, Sener A, Malaisse WJ: Hydrolysis of succinic acid dimethyl ester in rat pancreatic islets. Biochem Mol Med. 1995 Aug;55(2):131-7. Pubmed: 7582870 Link_out
              5. Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. Pubmed: 7729054 Link_out
              6. Groenen PM, Engelke UF, Wevers RA, Hendriks JC, Eskes TK, Merkus HM, Steegers-Theunissen RP: High-resolution 1H NMR spectroscopy of amniotic fluids from spina bifida fetuses and controls. Eur J Obstet Gynecol Reprod Biol. 2004 Jan 15;112(1):16-23. Pubmed: 14687733 Link_out
              7. Meijer-Severs GJ, van Santen E: Short-chain fatty acids and succinate in feces of healthy human volunteers and their correlation with anaerobe cultural counts. Scand J Gastroenterol. 1987 Aug;22(6):672-6. Pubmed: 3659829 Link_out
              8. Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. Pubmed: 12097436 Link_out
              9. Ren LC, Huang XY, Long JH: [Effects of succinic acid on the function of in vitro cultured human fibroblasts] Zhonghua Shao Shang Za Zhi. 2004 Feb;20(1):34-6. Pubmed: 15059451 Link_out
              10. Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. Pubmed: 8412012 Link_out
              11. Wevers RA, Engelke U, Heerschap A: High-resolution 1H-NMR spectroscopy of blood plasma for metabolic studies. Clin Chem. 1994 Jul;40(7 Pt 1):1245-50. Pubmed: 8013094 Link_out
              12. Borenstein DG, Gibbs CA, Jacobs RP: Gas-liquid chromatographic analysis of synovial fluid: volatile short-chain fatty acids in septic arthritis. Ann Rheum Dis. 1983 Aug;42(4):362-7. Pubmed: 6882030 Link_out
              13. Frenkel G, Peterson RN, Freund M: Oxidative and glycolytic metabolism of semen components by washed guinea pig spermatozoa. Fertil Steril. 1975 Feb;26(2):144-7. Pubmed: 1126459 Link_out
              14. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. Pubmed: 19212411 Link_out
              15. Briere JJ, Favier J, El Ghouzzi V, Djouadi F, Benit P, Gimenez AP, Rustin P: Succinate dehydrogenase deficiency in human. Cell Mol Life Sci. 2005 Oct;62(19-20):2317-24. Pubmed: 16143825 Link_out
              16. Rustin P, Rotig A: Inborn errors of complex II--unusual human mitochondrial diseases. Biochim Biophys Acta. 2002 Jan 17;1553(1-2):117-22. Pubmed: 11803021 Link_out

              Enzymes
              Name: Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
              Reactions:
              Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details
              Gene Name: SDHC
              Uniprot ID: Q99643 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
              Reactions:
              Succinic acid + Coenzyme Q10 unknown Fumaric acid + QH(2) details
              Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details
              Gene Name: SDHA
              Uniprot ID: P31040 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
              Reactions:
              Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details
              Gene Name: SDHD
              Uniprot ID: O14521 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
              Reactions:
              Succinic acid + Coenzyme Q10 unknown Fumaric acid + QH(2) details
              Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details
              Gene Name: SDHB
              Uniprot ID: P21912 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinate-semialdehyde dehydrogenase, mitochondrial
              Reactions:
              Succinic acid semialdehyde + NAD + Water unknown Succinic acid + NADH details
              Succinic acid semialdehyde + NAD + Water unknown Succinic acid + NADH + Hydrogen Ion details
              Gene Name: ALDH5A1
              Uniprot ID: P51649 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Phytanoyl-CoA dioxygenase, peroxisomal
              Reactions:
              Phytanoyl-CoA + Oxoglutaric acid + Oxygen unknown 2-Hydroxyphytanoyl-CoA + Succinic acid + CO(2) details
              Gene Name: PHYH
              Uniprot ID: O14832 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Prolyl 4-hydroxylase subunit alpha-2
              Reactions:
              L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details
              L-Proline + Oxoglutaric acid + Oxygen unknown 4-Hydroxyproline + Succinic acid + Carbon dioxide details
              Gene Name: P4HA2
              Uniprot ID: O15460 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Prolyl 4-hydroxylase subunit alpha-1
              Reactions:
              L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details
              L-Proline + Oxoglutaric acid + Oxygen unknown 4-Hydroxyproline + Succinic acid + Carbon dioxide details
              Gene Name: P4HA1
              Uniprot ID: P13674 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Gamma-butyrobetaine dioxygenase
              Reactions:
              4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen unknown L-Carnitine + Succinic acid + CO(2) details
              4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen unknown L-Carnitine + Succinic acid + Carbon dioxide details
              Gene Name: BBOX1
              Uniprot ID: O75936 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Procollagen-lysine,2-oxoglutarate 5-dioxygenase 1
              Reactions:
              L-lysine-[procollagen] + Oxoglutaric acid + Oxygen unknown (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2) details
              Protein lysine + Oxoglutaric acid + Oxygen unknown Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Water details
              Gene Name: PLOD1
              Uniprot ID: Q02809 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2
              Reactions:
              L-lysine-[procollagen] + Oxoglutaric acid + Oxygen unknown (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2) details
              Protein lysine + Oxoglutaric acid + Oxygen unknown Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Water details
              Gene Name: PLOD2
              Uniprot ID: O00469 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3
              Reactions:
              L-lysine-[procollagen] + Oxoglutaric acid + Oxygen unknown (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2) details
              Protein lysine + Oxoglutaric acid + Oxygen unknown Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Water details
              Gene Name: PLOD3
              Uniprot ID: O60568 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
              Reactions:
              Succinyl-CoA + a 3-oxo acid unknown Succinic acid + a 3-oxoacyl-CoA details
              Succinyl-CoA + Acetoacetic acid unknown Succinic acid + Acetoacetyl-CoA details
              Gene Name: OXCT1
              Uniprot ID: P55809 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinyl-CoA:3-ketoacid coenzyme A transferase 2, mitochondrial
              Reactions:
              Succinyl-CoA + a 3-oxo acid unknown Succinic acid + a 3-oxoacyl-CoA details
              Succinyl-CoA + Acetoacetic acid unknown Succinic acid + Acetoacetyl-CoA details
              Gene Name: OXCT2
              Uniprot ID: Q9BYC2 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
              Reactions:
              Guanosine triphosphate + Succinic acid + Coenzyme A unknown Guanosine diphosphate + Phosphoric acid + Succinyl-CoA details
              Adenosine triphosphate + Succinic acid + Coenzyme A unknown ADP + Phosphoric acid + Succinyl-CoA details
              Inosine triphosphate + Succinic acid + Coenzyme A unknown IDP + Phosphoric acid + Succinyl-CoA details
              Gene Name: SUCLG1
              Uniprot ID: P53597 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial
              Reactions:
              Guanosine triphosphate + Succinic acid + Coenzyme A unknown Guanosine diphosphate + Phosphoric acid + Succinyl-CoA details
              Inosine triphosphate + Succinic acid + Coenzyme A unknown IDP + Phosphoric acid + Succinyl-CoA details
              Gene Name: SUCLG2
              Uniprot ID: Q96I99 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial
              Reactions:
              Adenosine triphosphate + Succinic acid + Coenzyme A unknown ADP + Phosphoric acid + Succinyl-CoA details
              Guanosine triphosphate + Succinic acid + Coenzyme A unknown Guanosine diphosphate + Phosphoric acid + Succinyl-CoA details
              Inosine triphosphate + Succinic acid + Coenzyme A unknown IDP + Phosphoric acid + Succinyl-CoA details
              Gene Name: SUCLA2
              Uniprot ID: Q9P2R7 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Aspartyl/asparaginyl beta-hydroxylase
              Reactions:
              Peptide L-aspartate + Oxoglutaric acid + Oxygen unknown peptide 3-hydroxy-L-aspartate + Succinic acid + CO(2) details
              Gene Name: ASPH
              Uniprot ID: Q12797 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Hypoxia-inducible factor 1-alpha inhibitor
              Reactions:
              Hypoxia-inducible factor-L-asparagine + Oxoglutaric acid + Oxygen unknown hypoxia-inducible factor-(3S)-3-hydroxy-L-asparagine + Succinic acid + CO(2) details
              Ankyrin-repeat-L-histidine + Oxoglutaric acid + Oxygen unknown ankyrin-repeat-(3S)-3-hydroxy-L-histidine + Succinic acid + CO(2) details
              Gene Name: HIF1AN
              Uniprot ID: Q9NWT6 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Mitochondrial dicarboxylate carrier
              Reactions: Not Available
              Gene Name: SLC25A10
              Uniprot ID: Q9UBX3 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Solute carrier family 13 member 3
              Reactions: Not Available
              Gene Name: SLC13A3
              Uniprot ID: Q8WWT9 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: 17-beta-hydroxysteroid dehydrogenase type 6
              Reactions: Not Available
              Gene Name: HSD17B6
              Uniprot ID: O14756 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Solute carrier family 13 member 1
              Reactions: Not Available
              Gene Name: SLC13A1
              Uniprot ID: Q9BZW2 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Solute carrier family 13 member 2
              Reactions: Not Available
              Gene Name: SLC13A2
              Uniprot ID: Q13183 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Trimethyllysine dioxygenase, mitochondrial
              Reactions:
              N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown 3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine + Succinic acid + CO(2) details
              N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine + Succinic acid + Carbon dioxide details
              Gene Name: TMLHE
              Uniprot ID: Q9NVH6 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Alpha-ketoglutarate-dependent dioxygenase alkB homolog 2
              Reactions:
              DNA-base-CH(3) + Oxoglutaric acid + Oxygen unknown DNA-base + Formaldehyde + Succinic acid + CO(2) details
              Gene Name: ALKBH2
              Uniprot ID: Q6NS38 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Prolyl 3-hydroxylase 1
              Reactions:
              L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-3-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details
              Gene Name: LEPRE1
              Uniprot ID: Q32P28 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Prolyl 3-hydroxylase 2
              Reactions:
              L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-3-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details
              Gene Name: LEPREL1
              Uniprot ID: Q8IVL5 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Prolyl 3-hydroxylase 3
              Reactions:
              L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-3-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details
              Gene Name: LEPREL2
              Uniprot ID: Q8IVL6 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Prolyl 4-hydroxylase subunit alpha-3
              Reactions:
              L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details
              L-Proline + Oxoglutaric acid + Oxygen unknown 4-Hydroxyproline + Succinic acid + Carbon dioxide details
              Gene Name: P4HA3
              Uniprot ID: Q7Z4N8 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Lysine-specific demethylase 2A
              Reactions:
              Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details
              Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details
              Protein N6,N6-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown Protein lysine + Succinic acid + Formaldehyde + Carbon dioxide details
              Gene Name: KDM2A
              Uniprot ID: Q9Y2K7 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Lysine-specific demethylase 2B
              Reactions:
              Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details
              Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details
              Protein N6,N6-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown Protein lysine + Succinic acid + Formaldehyde + Carbon dioxide details
              Gene Name: KDM2B
              Uniprot ID: Q8NHM5 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Egl nine homolog 1
              Reactions:
              Hypoxia-inducible factor-L-proline + Oxoglutaric acid + Oxygen unknown hypoxia-inducible factor-trans-4-hydroxy-L-proline + Succinic acid + CO(2) details
              Gene Name: EGLN1
              Uniprot ID: Q9GZT9 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Egl nine homolog 2
              Reactions:
              Hypoxia-inducible factor-L-proline + Oxoglutaric acid + Oxygen unknown hypoxia-inducible factor-trans-4-hydroxy-L-proline + Succinic acid + CO(2) details
              Gene Name: EGLN2
              Uniprot ID: Q96KS0 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Egl nine homolog 3
              Reactions:
              Hypoxia-inducible factor-L-proline + Oxoglutaric acid + Oxygen unknown hypoxia-inducible factor-trans-4-hydroxy-L-proline + Succinic acid + CO(2) details
              Gene Name: EGLN3
              Uniprot ID: Q9H6Z9 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Transmembrane prolyl 4-hydroxylase
              Reactions:
              An HIF alpha chain L-proline + Oxoglutaric acid + Oxygen unknown An HIF alpha chain trans-4-hydroxy-L-proline + Succinic acid + CO(2) details
              Gene Name: P4HTM
              Uniprot ID: Q9NXG6 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Alkylated DNA repair protein alkB homolog 1
              Reactions:
              DNA-base-CH(3) + Oxoglutaric acid + Oxygen unknown DNA-base + Formaldehyde + Succinic acid + CO(2) details
              Gene Name: ALKBH1
              Uniprot ID: Q13686 Link_out
              Protein Sequence: FASTA
              Name: Lysine-specific demethylase 8
              Reactions:
              Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details
              Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details
              Gene Name: KDM8
              Uniprot ID: Q8N371 Link_out
              Protein Sequence: FASTA
              Name: Bifunctional lysine-specific demethylase and histidyl-hydroxylase MINA
              Reactions:
              L-histidine-[60S ribosomal protein L27a] + Oxoglutaric acid + Oxygen unknown (3S)-3-hydroxy-L-histidine-[60S ribosomal protein L27a] + Succinic acid + CO(2) details
              Gene Name: MINA
              Uniprot ID: Q8IUF8 Link_out
              Protein Sequence: FASTA
              Name: Bifunctional lysine-specific demethylase and histidyl-hydroxylase NO66
              Reactions:
              Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details
              Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details
              L-histidine-[60S ribosomal protein L8] + Oxoglutaric acid + Oxygen unknown (3S)-3-hydroxy-L-histidine-[60S ribosomal protein L8] + Succinic acid + CO(2) details
              Gene Name: NO66
              Uniprot ID: Q9H6W3 Link_out
              Protein Sequence: FASTA
              Name: Histone lysine demethylase PHF8
              Reactions:
              Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details
              Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details
              Gene Name: PHF8
              Uniprot ID: Q9UPP1 Link_out
              Protein Sequence: FASTA
              Name: Methylcytosine dioxygenase TET1
              Reactions:
              DNA 5-methylcytosine + Oxoglutaric acid + Oxygen unknown DNA 5-hydroxymethylcytosine + Succinic acid + CO(2) details
              Gene Name: TET1
              Uniprot ID: Q8NFU7 Link_out
              Protein Sequence: FASTA
              Name: Methylcytosine dioxygenase TET2
              Reactions:
              DNA 5-methylcytosine + Oxoglutaric acid + Oxygen unknown DNA 5-hydroxymethylcytosine + Succinic acid + CO(2) details
              Gene Name: TET2
              Uniprot ID: Q6N021 Link_out
              Protein Sequence: FASTA
              Name: Methylcytosine dioxygenase TET3
              Reactions:
              DNA 5-methylcytosine + Oxoglutaric acid + Oxygen unknown DNA 5-hydroxymethylcytosine + Succinic acid + CO(2) details
              Gene Name: TET3
              Uniprot ID: O43151 Link_out
              Protein Sequence: FASTA